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Dissecting the Origins of Sickle Cell Anemia
by Kathryn Norcutt Minority Nurse Writer
It was never fully explained during the movie Beasts of the Southern Wild, but we can surmise that Hushpuppy’s father may have suffered from sickle cell anemia. Her father, Wink, after being missing for days, finally strolls back to ‘The Bathtub’ still donned in his hospital gown and it is obvious something is seriously wrong with him. What, exactly, remains a mystery, but when Hushpuppy presents her father’s hospital identification bracelet to her schoolteacher, her teacher quickly gathers a type of folk-remedy to give to Hushpuppy that is supposed to help her father.
Of course, her father refuses his daughter’s ministrations, which only confounds Hushpuppy. Even after she faces the brutal aurochs, Hushpuppy still cannot prevent her father’s eventual demise. And as illustrated in real life, sickle cell is a very ruthless and painful genetic condition, most commonly found among the African American community.
According to the Centers for Disease Control and Prevention, it is estimated that 1 out of 12 African Americans have some form of the disease, either carrying the sickle cell trait, which generally has no symptoms but can be passed along to their subsequent offspring, or the full-blown case of it, which can entail a shortened lifespan as well as enduring days in a diminished capacity and longsuffering state. Tests are available that a pregnant mother can have performed during an amniocentesis to screen for sickle cell. Of course, if it comes back positive, she will still be faced with more questions than answers, among them treatment options, quality of life, and severity of the disease.
By investigating the origins of sickle cell anemia, we can understand how it went from one of the most shamefully neglected conditions to one promising scientific breakthrough, where perhaps one day, we will have something close to a cure for those future generations who are afflicted.
Interestingly enough, the sickle cell gene originated along the equatorial belt as a natural selective means to thwart the devastating effects of malaria. However, though it protects the carrier from mosquito-borne illness, sickle cell disease is a highly debilitating illness and its effects can diminish the stricken with a lifetime of painful joint, organ, and bone complications. In the human body, normal red blood cells are smooth-surfaced and round, hence the simplicity in which they slide through veins. When someone has full-blown sickle cell anemia, their hemoglobin is deficient, creating cells that are inherently sticky-surfaced and like a farming tool, sickle in shape. The problems caused by the damaged cells come in the form of chronic pain or ‘attacks’ where the cells have lodged together, either in certain organs, bones, or blood of the infirmed. The patient’s blood vessels are blocked by clumps of sticky, sickle cells, which is the source of the patient’s chronic pain.
This can range due to the severity of the disease and can consist of treating the pain with anti-inflammatories, or if needed, a bone marrow transplant. There have been some promising results with the use of Hydroxyurea, a drug commonly used in the treatment of cancer. It can help prevent the sickle cell buildup, reducing the amount and occurrence of painful attacks, but it has its side effects as well. Symptoms can include nausea, constipation, hair loss, fever, weight gain, leg ulcers, and bleeding. With children some experts are cautioning the use without knowing the long-term effects; some studies have demonstrated genetic changes and an increased risk for cancer. For those extreme cases there is bone marrow transplantation, but of course, this requires finding a compatible donor as well as extensive treatments.
Unfortunately, due to factors like medical discrimination, a lack of awareness, care, and treatment options, sickle cell anemia has historically not received the recognition among our scientific and medical community. On May 16th, 1972, President Richard Nixon took sickle cell anemia to center stage with the signing of the National Sickle Cell Anemia Control Act. But at the end of the day, it rests upon those in the medical community who can be that critical segue for pain management and controlling symptoms, especially young children who are suffering from the effects of this genetic disease. Treatment options, education, and cultural respect should be clearly understood for those caring for the afflicted.
Certainly, we have come a long way in recognizing the needs of a longsuffering community. And by implementing quality care, facilities, and adherence to treatment for those in the throes of this disease, we can undoubtedly help improve the overall quality of someone’s